Life-threatening pulmonary artery hypertension is rare and fraught with misconceptions, says Robert J. Schilz, DO, PhD, FCCP, Director of the Pulmonary Vascular Disease Program and Co-Director, Lung Transplantation at University Hospitals Cleveland Medical Center. But the problem of elevated pressure in the lungs is somewhat common, and has multiple etiologies.

Determining the true reason for the increased pressure is critical to the successful treatment of these patients.

Dr. Schilz explains, “All national and international guidelines recommend referring patients to a center that specializes in pulmonary artery hypertension for confirmation of diagnosis and ongoing management.

“Some of the advanced therapies are complex, including infusion, inhaled and other titratable medications. Having a partner to determine the appropriateness of treatment, and running these kinds of advanced therapies, is now the standard of care for patients.”

UH PULMONARY VASCULAR DISEASE PROGRAMPulmonary artery hypertension is a rare disease, and there are 14 medicines in five different classes, Dr. Schilz notes. “Cutting through who should be treated and how is a complex process,” he says. “Our clinic can help with that.”

The Pulmonary Vascular Disease Program at University Hospitals focuses on management of patients with diseases and processes that affect the pulmonary blood vessels. Dr. Schilz leads the pulmonary clinic, which he calls “a one-stop shop.”

The most common cause for elevated blood pressure is heart failure. So pulmonary hypertension cases make up the bulk of patient cases at the clinic. However, the program also sees patients with other lung-related blood vessel diseases, such as arterial venous malformations (AVM) and pulmonary emboli.

“AVMs can lead to low oxygen levels and disabilities,” Dr. Schilz says. “Sometimes they occur in conjunction with congenital or genetic problems, such as hereditary hemorrhagic telangiectasia.”

Fortunately, serious complications from AVMs, including death, are rare. But the clinic can help diagnose and then treat these patients with surgery, medications, embolization or radiation therapy.

For patients with pulmonary emboli, the clinic also offers potentially life-saving treatment. “We know that a small number of patients with blood clots in the lungs and its sequelae go on to suffer from chronic disability, dyspnea and an impaired ability to exercise or do things on a regular basis,” Dr. Schilz says. “The worst of them — about 3 to 4 percent of patients — ultimately develop chronic thromboembolic pulmonary hypertension, a life-threatening complication that arises from the inadequate resolution of acute blood clots.”

These patients will eventually require pulmonary thromboendarterectomy (PTE), a complex surgery that’s offered in only a dozen or so places in the country, Dr. Schilz explains. “The UH Pulmonary Vascular Disease Program is one of those places,” he notes.

CLINICAL RESOURCES AND EXPERTISEPhysicians throughout northeast Ohio can benefit from UH’s resources and expertise in pulmonary vascular disease management.

For example, the pulmonary clinic maintains a multi-disciplinary pulmonary embolism response team at the main UH campus that is available on a consultative basis. “We consult about the transfer of critically ill patients with pulmonary embolisms to evaluate them for advanced therapies, surgeries and other long-term management,” Dr. Schilz says.

UH also participates in all phases of clinical trials for treating pulmonary hypertension. In fact, it currently has three active trials using new and existing medications in novel ways, including treatment for pulmonary hypertension associated with intrinsic lung disease.

“Traditionally, these patients were not candidates for other treatments and it was unknown whether [alternative] treatments would be helpful for them,” Dr. Schilz says. “So at least one of these trials is helping answer this question.”

UH is rolling out new trials with novel agents, as well.

Despite advances, there is still a lot of misunderstanding and misdiagnosis of elevated blood pressure in the lungs, Dr. Schilz says. That is why the work being done at the UH Pulmonary Vascular Disease Program is so important.

“An accurate diagnosis is critical because the medications that are very useful for the rare kind of pulmonary hypertension can be ineffective — even counterproductive or downright dangerous — if you use them in the wrong groups of patients,” he says. “It is critical to understand what produces pulmonary hypertension, and that diagnosis and differential isn’t always easy. We’re here to help with that.”

To refer a patient to the Pulmonary Vascular Disease Program at UH, call 216-844-2639. UH staff will coordinate all the required information for a successful patient visit.

Call Direct 866-UH4-CARE (866-844-2273)or 800-552-8338

Contact a UH Physician Liaison Team Member Direct 216-286-9024

216-844-1111 or 800-421-9199

University Hospitals Cleveland Medical Center was formerly known as University Hospitals Case Medical Center. This portal includes some materials that were published prior to the name change on September 9, 2016.